Von Willebrand Disease profiling

Von Willebrand disease (vWD) is the most commonly inherited (autosomally) bleeding disorder, present at about 1% in the general population. It may affect males and females.

Von Willebrand Factor (vWF) is a multimeric protein produced in endothelial cells and megakaryocytes1. It circulates in blood as multimers ranging from 500 to more than 20,000 kDa. vWF mediates platelet adhesion to subendothelium of the damaged blood vessel and serves as a carrier for Factor VIII, extending its half-life into the bloodstream. 2,3

Ultra-large multimers are proteolytically cleaved by ADAMTS13 into smaller and less active vWF forms. The biological function of vWF depends largely on the size of its multimers. Larger multimers are more likely to bind to platelets and collagen, and to promote platelet adhesion in
circulating blood. 2,3

Von Willebrand disease is classified on the basis of criteria developed by the vWF Subcommittee of the ISTH, intending to be clinically relevant to the treatment of vWD. Diagnostic categories were defined that encompassed distinct pathophysiologic mechanisms and correlated with the response to treatment with DDAVP or blood products. 4,5

Type

Description

Quantitative

Type1

vWD is characterized by a partial quantitive deficiency of vWF (most frequent, about 70 to 80% of cases)

Type3

vWD is characterized by a severe qualitative deficiency of vWF (about 1 to 5% of cases)

Considering the heterogeneity of vWF concentration in the normal population, and in clinical manifestations, a complete biological study is necessary for vWD diagnosis, combining the assay of at least 3 parameters: FVIII:C activity, vWF activity (such as vWF:CBA or vWF RCo) and vWF antigen assay, and with regards to the ABO group and the familial context.

An Immunoturbidimetric assay for in vitro quantitative determination of von Willebrand Factor Antigen (vWF:Ag) on human citrated plasma, using a manuel of automated method. Reagents are in the liquid presentation, ready to use.

120206

R1: 4 x 5 ml

R2: 4 x 6 ml

ZYMUTESTTM vWF (CE-IVD)

An enzyme-immuno-assay for measuring human vWF in plasma, or in any fluid where vWF can be present.